Volume 82 | ESPE2014 | Next issue

ESPE 2014

Dublin, Ireland
18 Sep 2014 - 20 Sep 2014

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Dublin, Ireland; 18-20 September 2014. Further information

Poster Category 2

Puberty and Neuroendocrinology (1)

hrp0082p2-d2-541 | Puberty and Neuroendocrinology (1) | ESPE2014

Normal Minipuberty in a Patient with DAX1 Mutation: a Reliable Marker of the Function of the Hypothalamic–Pituitary–Gonadal Axis?

Fudvoye Julie , Christine Lebrethon Marie , Pierre Bourguignon Jean , Simone Parent Anne

Background: We report here the case of a 5-week-old male patient, referred to the hospital because of failure to thrive. An adrenal insufficiency was diagnosed and the genetic testing showed a mutation in the DAX1 gene leading to a premature stop codon.In addition to adrenal hypoplasia congenita, DAX1 mutation is known to be classically associated with hypogonadotrophic hypogonadism which is mostly characterized by absence of onset of puberty and inferti...

hrp0082p2-d2-542 | Puberty and Neuroendocrinology (1) | ESPE2014

GH Excess and Pseudoprecocious Puberty in a 8-Year-Old Boy with Mccune–Albright Syndrome

Ertl Diana-Alexandra , Gojo Johannes , Aubrunner Daniela , Haeusler Gabriele

Background: McCune–Albright syndrome (MAS) is defined by skin, bone and glands disorders, due to activating mutations in the GNAS1. Clinical presentation is heterogeneous. Reports about GH excess in MAS patients are scarce.Case report: We present the case of an 8-year-old male, previously diagnosed with mono-ostotic fibrous dysplasia of the skull, referred due to signs of pubertal development since the age of 6. The patient presented only 1...

hrp0082p2-d2-543 | Puberty and Neuroendocrinology (1) | ESPE2014

Time to Menarche After Completing GnRH Agonist in Girls with Central Precocious or Early Puberty

Kim Shin-Hee , Jung In-Ah , Cho Won Kyoung , Cho Kyoung Soon , Park So Hyun , Jung Min Ho , Suh Byoung Kyu

Background: Treatment goals for central precocious puberty (CPP) in girls include preventing short final height due to early epiphyseal closure, and avoiding premature onset of menarche.Objective and hypotheses: Our aim was to evaluate the timing of menarche and the associated factors among patients with idiopathic CPP or early-onset puberty (EP) who were treated with GnRH agonists (GnRHa).Method: We analyzed clinical and laborator...

hrp0082p2-d2-544 | Puberty and Neuroendocrinology (1) | ESPE2014

Impact of Being Born Small or Large for Gestational Age on Pubertal Onset and Progression

Loredana Marcovecchio M , Di Giovanni Ilaria , de Giorgis Tommaso , Polidori Nella , Chiavaroli Valentina , Mohn Angelika

Background: Perinatal factors seem to influence the onset of puberty. There is some evidence suggesting that being born small for gestational age (SGA) is associated with early puberty, whereas the effect of large size at birth on timing of puberty is not clear.Objective and hypotheses: To evaluate the timing of puberty in children born SGA and large for gestational age (LGA) compared to children born appropriate for gestational age (AGA).<p class="a...

hrp0082p2-d2-545 | Puberty and Neuroendocrinology (1) | ESPE2014

Diagnostic Spectrum of Female Pubertal Delay

Uday Suma , Alvi Sabah

Introduction: Delayed onset of puberty is quite a common presentation in adolescent endocrine clinics, and the most common cause, particularly in boys is considered to be constitutional delay of growth and maturation. In girls, however, it is more likely that there is a significant underlying problem.Objectives: To review the aetiology of pubertal delay in female patients referred to a single tertiary centre.Methods: All female pat...

hrp0082p2-d2-546 | Puberty and Neuroendocrinology (1) | ESPE2014

Kallmann Syndrome: Diagnosis in Paediatric Age

Machado Angela , Oliveira Maria Joao , Borges Teresa , Cardoso Helena , Fonseca Paula , Ribeiro Luis , Goncalves Catarina , Lemos Manuel

Background: Kallmann syndrome (KS) is a rare clinical entity, characterized by the association of hipogonadotropic hypogonadism and hypo/anosmia, with an estimated prevalence of 1:8000 in males and 1:40 000 in females.Method: Retrospective study of cases of KS diagnosed in paediatric age. Genetic analysis was performed by PCR and DNA sequencing of KAL1, FGFR1, GNRHR, GNRH1, PROK2, PROKR2, KISS1R, TAC3, TACR3, and FGF8 genes.Results...

hrp0082p2-d2-547 | Puberty and Neuroendocrinology (1) | ESPE2014

The Triptorelin Test Compares Favourably with the GnRH Test in the Diagnosis of Central Precocious Puberty

Ladjouze Asmahane , Djermane Adel , Ouarezki Yasmine , Kedji Leila , Berkouk Karima , Abdeljalil Maoudj , Aboura Rawda , Bensmina Minoubia , Anane Tahar , Bouyoucef Salah Eddine , Laraba Abdenour

Background: The i.v. GnRH test remains the gold standard for the diagnosis of central precocious puberty (CPP). Unfortunately however, GnRH is expensive and is not available worldwide. GnRH analogues have been used as an alternative, but their place is not established, while very few studies have compared between the two tests.Objective: To compare the effects of GnRH and Triptorelin on gonadotrophin secretion in patients with sexual precocity and hence ...

hrp0082p2-d2-548 | Puberty and Neuroendocrinology (1) | ESPE2014

Puberty in Children with Shunted Congenital Hydrocephalus with and without Myelomeningocele

Proos Lemm , Arnell Kai , Gustafsson Jan , Dahl Margareta

Background: Children with myelomeningocele (MMC) run an increased risk of developing early or precocious puberty (E/PP). In previous studies of such children we found that the incidence of E/PP was 52% in girls and 21% in boys and that E/PP was strongly associated with increased intracranial pressure perinatally. It is also known that the occurrence of hydrocephalus without MMC is associated with risk of E/PP.Objective and hypotheses: The aim was to stud...

hrp0082p2-d2-549 | Puberty and Neuroendocrinology (1) | ESPE2014

Evaluation of Age at GnRH Analogue Treatment Discontinuation, Age at Menarche and Adult Height in Girls with Central Precocious Puberty from the Spanish Registry

Corripio Raquel , Soriano-Guillen Leandro , Canete Ramon , Castro-Feijoo Lidia , Escribano Arancha , Espino Rafael , Herrero-Espinet Javier , Labarta Jose-Ignacio , Argente Jesus

Background: The Spanish Registry PUBERE was created (2007) with 53 hospitals.Objective and hypotheses: i) To determine the chronological age (CA) and bone (BA) at GnRH analogue withdrawal; ii) to analyze the age at menarche and time elapsed after stopping treatment; and iii) to know adult height data.Method: Patients with central precocious puberty (CPP) born after 1992, diagnosed before 8 years in girls, with BA/CA >1 year and...

hrp0082p2-d2-550 | Puberty and Neuroendocrinology (1) | ESPE2014

Final Height in a Boy with McCune–Albright Syndrome and Precocious Puberty Treated with Ketoconazole, Cyproterone Acetate, and Leuprolide Acetate Depot for More than 5 Years

Messina Maria Francesca , Aversa Tommaso , Valenzise Mariella , De Luca Filippo

Background: McCune–Albright syndrome (MAS) is a sporadic and rare disorder, clinically defined by the classic triad of cafè-au-lait skin lesions, polyostotic fibrous dysplasia, and peripheral precocious puberty. Precocious puberty is common in girls, but has been reported in only 15% of affected boys. Clinical trials on therapeutic management of precocious puberty in MAS boys are limited to case reports or small patient cohorts and no data are reported about final he...

hrp0082p2-d2-551 | Puberty and Neuroendocrinology (1) | ESPE2014

The Effect of Combined GH and GnRH Agonist Treatment for Near-Final Height Improvement in True Precocious Puberty and Early Puberty

Kim Se Young , Kim Eun Young , Yi Kyung Hee

Purpose: The goal of treatment for true precocious puberty (TPP) and early puberty with GnRH agonist (GnRHa) is to prevent loss of genetic potential of target height (TH). And to regress secondary sex characteristics appropriate for patient’s age. But some patient’s growth velocity (GV) after treatment would decline and suggest that final height (FH) was not improved. So, we investigated the effect of combined GH and GnRHa treatment for near-FH (NFH) improvement....

hrp0082p2-d2-552 | Puberty and Neuroendocrinology (1) | ESPE2014

Pubertal Development in a Cohort of Romanian School-Aged Children

Pascanu Ionela , Capraru Oana , Cota Ancuta , Pop Raluca

Background: The average age of onset of puberty development has lowered in last decades due to multiple confounding factors. No recent populational studies are available in our country regarding pubertal development.Objective and hypotheses: The aim of our study was to identify the timing of pubertal characteristics in our region in children of school age. Our hypothesis was that the age of pubertal onset has diminished.Method: Typ...